(53) Pregnancies in women with cystic fibrosis – with or without the intake of elexacaftor/ tezacaftor/ ivacaftor

Background: Today, highly effective modulators are available for the majority of patients with cystic fibrosis (CF). Especially the triple combination elexacaftor/ tezacaftor/ ivacaftor (ETI) caused a great improvement in health. An increase in pregnancy rates has been described. According to the prescribing information, the intake of ETI is not recommended during pregnancy. We want to report about 11 pregnancies with or without ETI.

Methods: This is a cohort study of all pregnancies at our Cystic Fibrosis Center for of Adults between September 2020 and March 2022.

Results: Until now, 11 pregnancies have been reported. Mean age was 33.7 years, mean FEV1 was 74.1% (range 34.5%- 123.0%). There was no miscarriage. 6 women decided to continue with ETI, one woman started with ETI in week 25 as lung function deteriorated, one woman decided to discontinue ETI after the diagnosis of pregnancy. 3 women had no exposure of ETI at all (2 women didn’t start with ETI because of their planned pregnancy, 1 woman isn’t eligible for modulator therapy). In the 7 women on ETI, no intravenous (IV) therapy was necessary during pregnancy. Lung function remained stable or increased during pregnancy (mean increase in FEV1: 12,6.6%, range 5,8%-25%). In the 4 women without ETI, 3 cycles of iv-therapy were needed due to a decrease in FEV1 (2 cycles of iv-therapy in 1 woman, 1 cycle of iv-therapy in another woman during pregnancy with a second cycle needed immediately after delivery). These women had a loss of FEV1 of 1.6% and 2,5% during their pregnancies. There were two maternal complication: one woman on ETI had a placenta previa, and one woman without ETI had a pulmonary embolism after sectio caeserea. 7 babies have been delivered until now (4 sectio caesarea, 3 spontanous deliveries) with 3 babies born before week 38. All babies are alive with no signs of malformation. There were two complications in the 7 children (1 sepsis in a baby of a women not exposed to ETI, and one baby with breathing difficulties due to preterm delivery in a woman on ETI). No complication was referred to the intake of ETI.

Conclusions: Pregnant women who decide to continue the intake of ETI seem to have a better course of pregnancy with an increase in lung function and less need for iv-therapy. No therapy-related complications have been reported until now.

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